Rhabdomyosarcoma in children: Retrospective analysis from a single tertiary care center in Saudi Arabia
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Published on: October 14, 2016
Samer N Markiz1, Safia Khan2, Zainab B Wagley3
1Department of Pediatric Hematology/Oncology, King Faisal Specialist Hospital & Research Centre, Riyadh, Saudi Arabia.
Insights
Pediatric rhabdomyosarcoma (RMS) outcomes in Saudi Arabia show 5-year survival rates of 64.3% overall and 53.3% event-free. Advanced disease and unfavorable factors challenge treatment, necessitating improved diagnostics and national collaboration.
Area of Science:
- Pediatric Oncology
- Sarcoma Research
- Clinical Outcomes
Background:
- Rhabdomyosarcoma (RMS) is the most common pediatric soft tissue sarcoma, frequently affecting the head and neck.
- Multimodality treatment (chemotherapy, surgery, radiation) has improved localized disease survival, but advanced stages remain challenging.
- This study examines clinical characteristics and outcomes of RMS patients treated at a Saudi Arabian tertiary care center.
Purpose of the Study:
- To report the clinical characteristics and treatment outcomes of pediatric rhabdomyosarcoma patients.
- To identify factors influencing survival in children and adolescents with RMS.
- To provide insights into current management strategies and identify areas for improvement.
Main Methods:
- Retrospective analysis of 124 pediatric patients (0-14 years) diagnosed with RMS between 2005 and 2018.
- Statistical analysis using SPSS, including Kaplan-Meier for survival and Cox proportional hazards for multivariate analysis.
- Evaluation of primary tumor site, histology, clinical risk group, and initial response to therapy.
Main Results:
- The most common primary sites were head/neck (30%) and genitourinary (25%). Embryonal RMS predominated (81%).
- Five-year overall survival was 64.3%, and event-free survival was 53.3%.
- Survival was significantly influenced by primary tumor site, histology, and clinical risk group. Unfavorable factors predicted poorer outcomes.
Conclusions:
- The study offers insights into current rhabdomyosarcoma management outcomes in the region.
- Incorporating cytogenetics and molecular diagnostics into standard care is recommended.
- National collaborative efforts are crucial for improving pediatric RMS outcomes.
Background:
Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children occurring most commonly in the head and neck region. The treatment involves using a multimodality approach including chemotherapy, surgery, and radiation therapy. Survival for patients with localized disease has improved markedly, but the treatment of advanced disease remains a challenge. We report the clinical characteristics and outcome for patients treated at a tertiary care center in Saudi Arabia.
Methods:
Patients aged 0-14 years diagnosed with RMS between 2005 and 2018 were included. Statistical analysis was performed using SPSS software. Kaplan-Meier method was used to calculate overall and event free survival. Cox proportional hazards model was used for multivariate analysis.
Results:
One hundred and twenty-four patients were analyzed. The median age was 5.7 years with male predominance (2.4:1). The most common primary sites were head/neck (30%) and the genitourinary tract (25%). Embryonal RMS was present in 81%; alveolar in 19%. Most patients had intermediate risk disease (60%). The 5-year overall and event free survivals were 64.3% and 53.3%, respectively. Survival was influenced by primary tumor site, histology, and clinical risk group. Unfavorable primary site, high risk stratification, and poor initial response to therapy predicted a poor outcome.
Conclusion:
This study provides an insight on the current management outcomes for our patients with RMS. Cytogenetics and molecular diagnostics need to be incorporated as standard of care in the therapeutic approach of our patients. In addition, there is a need for national collaborative efforts to improve the outcome of RMS in children and adolescents.
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